And Review of Acute nephritis Syndromes

Karyomegalic Tubulointerstitial Nephritis Symptoms:

Recurrent PneumoniasRenal failure leading invariably to ESRDHypertensionHematuriaTypically patients present in third decade of

life.

Karyomegalic Tubulointerstitial Nephritis Clinical features:

Negative seriologies for autoimmune and viral causes

Pathology shows:○ Interstitial fibrosis○ Large nuclei in proximal tubular cells with odd

shape and focal intra-nuclear clearing○ Negative for immunoflorescence○ Irregular nuclear membranes on electron

microscopy

Karyomegalic Tubulointerstitial Nephritis Etiology: believed to be secondary to a

mitotic block Associated with HLA A9/B35 genotype Viral Etiologies have mostly been

debunked due to negative seriologies Chemical exposure is not a consistent

trademark

Acute Nephritis Syndromes

Post Strep GN:○ Happens after skin or throat infection with Beta

hemolytic Strep particulary type M, 1-3 weeks after throat or 2-6 weeks after skin infection

○ Acute endocapillary proliferatie GNDx: symptoms of hematuria, edema, nephrotic

range proteinuria, headache, hypertension, oliguria○ Antistrep antibodies positive○ Renal biopsy showing PMNs, glomerular immune

deposits of IGG, IGM, C3, C4, and C5-9 (humps) Rx: supportive.

Acute Nephritis Syndromes

Lupus nephritis:○ Symptoms: hematuria, hypertension, renal failure○ WHO classifications:

I: normalII: mesanigal immune complexes with mesangial

proliferationIII- IV: focal to global diffuse proliferative disease

- III: steroids, IV: steroids mycofenalate or cyclophosphamide.

V: bubendothelial immune deposits (membranous pattern, - -steroids

Remember: full house: of IG’s and positive Anti-ds DNA antibodies.

Acute Nephritis Syndromes Anti Basement membrane disease

Hemorrhage and glomerulonephritis, oliguria○ Associated with pulmonary hemorrhage

(goodpastures disease)DX: Anti-GMB antibodies (IgG rarely IgA)

○ Biopsy with smooth glomerular staining.○ ANCAs may be positive with goodpastures

syndrome (myeloperoxidaseRx: Plasmapheresis, steroids,

Acute Nephritis Syndromes IgA Nephropathy

Dx: <20 years old typically, preceding infection, hematuria, Deposition of IgA in mesangium.○ One of most common GN worldwide○ Common in asians and souther europeans (30

and 20% prevalance respectively)Rx: ACE inhibitors, tonsilectomy, steroids,

○ If RPGN: steroids, cytotoxic agents, plasmapheresis

References

Harrisons internal medicine Baba et al, karyomegalic tubulointerstital

nephritis – a case report, Pathology, research and practice, 202 2006 555-559.